Causes of Cushing’s Syndrome

Posted on October 2, 2016 by MaryO

Cushing’s syndrome—also referred to as hypercortisolism—is fairly rare. However, researchers have boiled down a few key causes of Cushing’s syndrome, which you’ll read about below.

The cause of Cushing’s syndrome boils down to: Your body is exposed to too much cortisol. There are a few ways that this over-exposure can happen, including taking certain medications and having a tumor on your pituitary gland or adrenal gland.

Can Taking Corticosteroids Cause Cushing’s Disease?
One particular type of medication can cause Cushing’s syndrome: corticosteroids. But rest assured: Not all steroid medications cause Cushing’s syndrome. It’s more common to develop Cushing’s syndrome from steroids you take in pill form or steroids you inject. Steroid creams and steroids you inhale are not common causes of Cushing’s syndrome.

Some steroid medications have the same effect as the hormone cortisol does when produced in your body. But as with an excessive production of cortisol in your body, taking too much corticosteroid medications can, over time, lead to Cushing’s syndrome.

It’s common for people with asthma, rheumatoid arthritis, and lupus to take corticosteroids. Prednisone (eg, Deltasone) is an example of a corticosteroid medication.

Other Cushing’s Disease Causes
Your body can over-produce cortisol or adrenocorticotropic hormone (ACTH). The pituitary gland secretes ACTH, which is in charge of stimulating the adrenal glands to produce cortisol, and the adrenal glands are responsible for releasing cortisol into the bloodstream.

Cortisol performs important tasks in your body, such as helping to maintain blood pressure and regulate how your body metabolizes proteins, fats, and carbohydrates, so it’s necessary for your body to maintain normal levels of it.

The following can cause excessive production of cortisol or ACTH, leading to Cushing’s syndrome.

  • Pituitary gland tumors: A benign (non-cancerous) tumor of the pituitary gland can secrete an excess amount of ACTH, which can cause Cushing’s syndrome. Also known as pituitary adenomas, benign tumors of the pituitary gland affect women 5 times more often than men.
  • Adrenal gland tumors: A tumor in one of your adrenal glands can lead to Cushing’s syndrome by causing too much cortisol to enter your bloodstream. Most of these tumors are non-cancerous (called adrenal adenomas).

    Cancerous adrenal tumors—called adrenocortical carcinomas—are relatively rare. These types of tumors typically cause extremely high levels of cortisol and very rapid development of symptoms.

  • Other tumors in the body: Certain tumors that develop outside the pituitary gland can also produce ACTH. When this happens, it’s known as ectopic ACTH syndrome. Ectopic means that something is in an abnormal place or position. In this case, only the pituitary gland should produce ACTH, so if there is a tumor producing ACTH and it isn’t located on the pituitary, it’s ectopic.

    It’s unusual to have a tumor that secretes ACTH outside the pituitary. These tumors are usually found in the pancreas, lungs, or thyroid, and they can be benign or malignant (cancerous).

    The most common forms of ACTH-producing tumors are small cell lung cancer, which accounts for about 13% of all lung cancer cases, and carcinoid tumors—small, slow-growing tumors that arise from hormone-producing cells in various parts of the body.

  • Familial Cushing’s syndrome: Although it’s rare, Cushing’s syndrome can develop from an inherited tendency to have tumors on one or more of your endocrine glands. Some inherited conditions, such as multiple endocrine neoplasia (MEN 1), can involve tumors that over-produce cortisol or ACTH, leading to Cushing’s syndrome.

If you think you could have Cushing’s syndrome or you have questions about the causes of Cushing’s syndrome, talk to your doctor immediately.

Written by | Reviewed by Daniel J. Toft MD, PhD, adapted from  http://www.endocrineweb.com/conditions/cushings-syndrome/cushings-syndrome-causes

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Delayed diagnosis, barriers to care increase morbidity in children with Cushing’s syndrome

Posted on May 6, 2016 by MaryO

Hispanic and black children diagnosed with Cushing’s syndrome are more likely to present with higher cortisol measurements and larger tumor size vs. white children, according to study findings presented at the annual Pediatric Academic Societies Meeting in Baltimore.

“Racial and socioeconomic disparities may contribute to the severity of disease presentation for children with Cushing’s [syndrome],” Alexandra Gkourogianni, MD, of the section on endocrinology and genetics at the Eunice Kennedy Shriver National Institute of Child Health and Human Development, and colleagues wrote. “Minority children from disadvantaged backgrounds present more frequently with comorbidities associated with longstanding [Cushing’s syndrome].”

Gkourogianni and colleagues analyzed data from 135 children treated for Cushing’s syndrome (transsphenoidal surgery) at the NIH between 1997 and 2015 (mean age, 13 years; 51% girls; 33% Hispanic or black). Researchers used a 10-point index for rating severity in pediatric Cushing’s syndrome based on predefined cutoffs; degree of hypercortisolemia, impaired glucose tolerance, and hypertension were graded on a 3-point scale (0-2); height, BMI z scores, duration of disease, and tumor invasion were graded on a 2-point scale (0-1).

Researchers found that midnight cortisol measurements were higher among Hispanic and black children vs. white children (23.3 µg/dL vs. 16 µg/dL; P = .019), as were tumor sizes (mean 6.3 mm vs. 3.3 mm; P = .016). Height standard deviation score was more severely affected in black and Hispanic children (–1.6 vs. –1.1; P = .038), and mean Cushing’s syndrome score for Hispanic and black children was higher vs. white children (4.5 vs. 3.8; P = .033).

Researchers found that median income had an independent correlation with Cushing’s syndrome score in univariate regression analysis for covariates of socioeconomic status and demographics (P = .025). Multivariable regression analysis using race, prevalence of obesity, estimated income, access to pediatric endocrinologist, age and sex confirmed that race, along with lower socioeconomic status and older age, were predictors of a higher Cushing’s syndrome score (P = .002).

“We speculate that delayed diagnosis, barriers to access to care and poorer quality health care for these underserved patients may contribute to presentation at a later age and increased morbidity,” the researchers wrote. “Additional research is needed to identify potential modifiable factors that may improve care for these patients.” – by Regina Schaffer

Reference:

Gkourogianni A, et al. Poster #445. Presented at: Pediatric Academic Societies Meeting; April 30- May 3, 2016; Baltimore.

Disclosure: Endocrine Today was unable to determine relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7Be79d7c84-d539-4a04-a548-882b9f4caadd%7D/delayed-diagnosis-barriers-to-care-increase-morbidity-in-children-with-cushings-syndrome

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Young people with Cushing syndrome may be at higher risk for suicide, depression

Posted on March 30, 2016 by MaryO

Children with Cushing syndrome may be at higher risk for suicide as well as for depression, anxiety and other mental health conditions long after their disease has been successfully treated, according to a study by researchers at the National Institutes of Health.

Cushing syndrome results from high levels of the hormone cortisol. Long-term complications of the syndrome include obesity, diabetes, bone fractures, high blood pressure, kidney stones and serious infections. Cushing’s syndrome may be caused by tumors of the adrenal glands or other parts of the body that produce excess cortisol. It also may be caused by a pituitary tumor that stimulates the adrenal glands to produce high cortisol levels. Treatment usually involves stopping excess cortisol production by removing the tumor.

“Our results indicate that physicians who care for young people with Cushing syndrome should screen their patients for depression-related mental illness after the underlying disease has been successfully treated,” said the study’s senior author, Constantine Stratakis, D(med)Sci, director of the Division of Intramural Research at NIH’s Eunice Kennedy Shriver National Institute of Child Health and Human Development. “Patients may not tell their doctors that they’re feeling depressed, so it’s a good idea for physicians to screen their patients proactively for depression and related conditions.”

Cushing syndrome may affect both adults and children. A recent study estimated that in the United States, there are 8 cases of Cushing syndrome per 1 million people per year.

The researchers published their findings in the journal Pediatrics. They reviewed the case histories of all children and youth treated for Cushing syndrome at NIH from 2003 to 2014, a total of 149 patients. The researchers found that, months after treatment, 9 children (roughly 6 percent) had thoughts of suicide and experienced outbursts of anger and rage, depression, irritability and anxiety. Of these, 7 experienced symptoms within 7 months of their treatment.

Two others began experiencing symptoms at least 48 months after treatment.

The authors noted that children with Cushing syndrome often develop compulsive behaviors and tend to become over-achievers in school. After treatment, however, they then become depressed and anxious. This is in direct contrast to adults with Cushing syndrome, who tend to become depressed and anxious before treatment and gradually overcome these symptoms after treatment.

The authors stated that health care providers might try to prepare children with Cushing syndrome before they undergo treatment, letting them know that their mood may change after surgery and may not improve for months or years. Similarly, providers should consider screening their patients periodically for suicide risk in the years following their treatment.

Source: NIH/Eunice Kennedy Shriver National Institute of Child Health and Human Development

From http://www.news-medical.net/news/20160329/Young-people-with-Cushing-syndrome-may-be-at-higher-risk-for-suicide-depression.aspx

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Johns Hopkins surgeon ‘Dr. Q’ to get Hollywood treatment

Posted on March 9, 2016 by MaryO

DrQ

 

Brad Pitt’s production company Plan B has teamed up with Disney to develop a movie based on the life of Alfredo Quiñones-Hinojosa, the head of brain tumor surgery at Johns Hopkins Hospital.

Quiñones-Hinojosa’s path to becoming a physician started in an unlikely place: a cotton field. He had come to the United States in 1987 from his native Mexico at the age of 19, penniless and unable to speak English. Driven to have a better life than the one he would have had in Mexico, he took jobs picking cotton, painting, and welding to pay for his tuition at San Joaquin Delta Community College in Stockton, California.

“These very same hands that now do brain surgery, right around that time they had scars everywhere from pulling weeds. They were bloody,” he told CNN correspondent Sanjay Gupta in a 2012 interview.

After earning his medical degree from Harvard Medical School and training in both general surgery and neurosurgery at the University of California, San Francisco, Quiñones-Hinojosa came to Johns Hopkins in 2005 and became a faculty member and surgeon. He specializes in brain cancer and pituitary tumors. His autobiography Becoming Dr. Q: My Journey from Migrant Farm Worker to Brain Surgeon was published in 2011 and received the International Latino Book Award in 2012.

Feeling like an outsider helped keep Quiñones-Hinojosa focused and “at the top of his game,” he told CNN. In the keynote speech delivered at Johns Hopkins University’s 2013 commencement ceremony, he elaborates, weaving together memories of his own brush with death in a work accident with his experience operating on a patient with a massive brain tumor that unexpectedly ruptured during surgery. Quoting the migrant farm worker and civil rights activist Cesar Chavez, he says, “If you are afraid, you will work like crazy.”

Plan B began developing the project—titled Dr. Q, the nickname for Quiñones-Hinojosa adopted by his patients—in 2007 after hearing a radio broadcast about the doctor and his background.

Matt Lopez, author of the popular Civil War play The Whipping Man and a former staff writer for HBO’s The Newsroom, will write the script.

According to The Hollywood Reporter, Disney expects Dr. Q to be a modestly-budgeted inspirational drama. Plan B executives Pitt, Dede Gardner, and Jeremy Kleiner won Best Picture Oscars two years ago for their production work on 12 Years a Slave and were nominated this year for their work on The Big Short.

From http://hub.jhu.edu/2016/03/07/brad-pitt-disney-dr-q-movie

 

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Pituicytoma and Cushing’s Disease in a 7-Year-Old Girl: A Mere Coincidence?

Posted on November 9, 2015 by MaryO

Paola Cambiaso, Donato Amodio, Emidio Procaccini, Daniela Longo, Stefania Galassi, Francesca Diomedi Camassei, Marco Cappa

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Abstract

Pituicytoma is a tumor extremely rare in childhood, with only 4 cases reported in literature. It is thought to arise from the specialized glial elements called “pituicytes.” The association of pituicytoma and Cushing’s disease (CD) has been described only once so far, in an adult patient.

A 7-year-old girl was referred for clinical signs of hypercortisolism, and a diagnosis of CD was made. MRI revealed 2 pathologic areas in the pituitary gland. The patient underwent surgery, with microscopic transsphenoidal approach, and a well-circumscribed area of pathologic tissue was identified and removed. Surprisingly, histologic and immunohistochemical study provided unequivocal evidence of pituicytoma. No pituitary adenoma could be identified.

For persistent hypercortisolism, the patient necessitated transsphenoidal endoscopic reintervention and 2 other lesions were removed. By immunohistological examination, these lesions were confirmed to be corticotropin-secreting adenoma. Unfortunately, there was no postoperative decrease in corticotropin and cortisol levels, and the patient underwent bilateral laparoscopic adrenalectomy.

Considering that we report a second case of association of pituicytoma and corticotropin-secreting adenoma, that CD is infrequent, and pituicytoma is extremely rare in childhood, the coexistence of these 2 tumors should not be considered a mere coincidence. To date, there is no conclusive evidence about the origin of these different subtypes of pituitary tumors. This case supports the hypothesis that these tumors share a common progenitor cell, which could be the folliculostellate cell.

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